Overview
Definition:
Spastic diplegia is the most common form of cerebral palsy (CP), characterized by increased muscle tone (spasticity) primarily affecting the legs, leading to stiff or "scissoring" gait
It is a non-progressive disorder of the brain's motor control centers, often resulting from prenatal or perinatal insults.
Epidemiology:
Spastic CP accounts for approximately 70-80% of all CP cases
The incidence of CP is estimated to be around 2-3 per 1000 live births globally, with variations based on geographic location, socioeconomic factors, and access to prenatal care
Spastic diplegia specifically has a higher prevalence in premature infants and those with a history of hypoxic-ischemic encephalopathy.
Clinical Significance:
Spastic diplegia significantly impacts a child's mobility, independence, and participation in daily activities
Effective management with orthoses and gait training is crucial for improving functional outcomes, preventing secondary complications like contractures and deformities, and enhancing the quality of life for affected children and their families
This topic is frequently tested in DNB and NEET SS examinations.
Clinical Presentation
Symptoms:
Delayed motor milestones, such as sitting, crawling, and walking
Difficulty walking, with an unsteady or "toe-walking" gait
Leg stiffness, particularly in the hips, knees, and ankles
Scissoring of the legs during walking
Clumsiness and poor balance
Increased fatigue during physical activities
Occasional speech and swallowing difficulties (dysarthria, dysphagia) depending on associated brain lesions.
Signs:
Hypertonia (spasticity), predominantly in lower extremities, manifesting as increased resistance to passive movement
Exaggerated deep tendon reflexes (hyperreflexia)
Clonus at the ankles
Positive Babinski sign
Hip adductor and hamstring tightness
Equinus (plantarflexed) or calcaneovalgus (dorsiflexed) deformities of the feet
A characteristic gait pattern: crouched, toe-walking, with increased stance phase and decreased swing phase, and possible adductor spasticity causing circumduction or scissoring.
Diagnostic Criteria:
Diagnosis is primarily clinical, based on the presence of persistent, non-progressive motor deficits, particularly spasticity, and a history suggestive of a brain insult occurring before, during, or shortly after birth
Neuroimaging (MRI brain) can help identify the location and extent of brain lesions consistent with the clinical presentation, though it is not always necessary for diagnosis.
Diagnostic Approach
History Taking:
Detailed prenatal, perinatal, and postnatal history, including risk factors for brain injury (e.g., prematurity, low birth weight, maternal infections, placental insufficiency, birth asphyxia, neonatal sepsis, meningitis, stroke)
Developmental milestones and gross motor function assessment
Family history of neurological disorders
Information regarding previous interventions and their effectiveness
Red flags: persistent primitive reflexes, asymmetrical tonic neck reflex beyond 6 months, significant head lag beyond 4 months.
Physical Examination:
Comprehensive neurological examination focusing on motor tone (spasticity, rigidity), reflexes (deep tendon, superficial), muscle strength, coordination, and balance
Assessment of posture and gait
Evaluation of joint range of motion to identify contractures and deformities
Examination of the hips for subluxation or dislocation
Screening for associated sensory, cognitive, visual, and auditory impairments.
Investigations:
Magnetic Resonance Imaging (MRI) of the brain is the gold standard for identifying structural abnormalities such as periventricular leukomalacia, basal ganglia lesions, or cortical damage
Electroencephalogram (EEG) may be used to rule out or monitor for seizures
Genetic testing is rarely indicated unless a specific genetic syndrome is suspected
Metabolic screening may be considered in atypical cases.
Differential Diagnosis:
Other forms of cerebral palsy (dyskinetic, ataxic, mixed), hereditary spastic paraplegia (HSP), spinal muscular atrophy (SMA), muscular dystrophies, Leigh syndrome, hereditary metabolic disorders, metabolic encephalopathies, metabolic myopathies, benign hereditary chorea, tethered cord syndrome, spinal cord tumors, and certain genetic syndromes that mimic CP symptoms.
Management
Initial Management:
Early identification and referral to a multidisciplinary team
Establishing baseline functional assessment and setting realistic goals
Initiating a personalized intervention plan incorporating physical therapy, occupational therapy, and orthotic management.
Orthotic Management:
Ankle-foot orthoses (AFOs) are crucial for managing equinus deformities and improving gait stability
Static AFOs provide support and limit plantarflexion, often used for toe-walking
Hinged AFOs allow for more natural dorsiflexion during the swing phase of gait
Dynamic AFOs can assist with push-off
Proper fitting, material selection (e.g., carbon fiber, thermoplastic), and periodic adjustments are essential
The goal is to promote a more efficient and stable gait, reduce energy expenditure, and prevent secondary deformities.
Gait Training:
Gait training is a cornerstone of rehabilitation, focusing on improving walking quality, balance, and endurance
It involves therapeutic exercises, functional mobility training, and the use of assistive devices (e.g., walkers, canes)
Techniques include task-specific training, motor learning principles, and incorporating dynamic activities
Emphasis is placed on achieving optimal biomechanics, reducing compensatory movements, and maximizing independence
This often includes proprioceptive neuromuscular facilitation (PNF), constraint-induced movement therapy (CIMT) adaptations, and task-oriented training.
Medical Management:
Pharmacological management of spasticity may include oral medications such as baclofen (oral or intrathecal), tizanidine, and dantrolene sodium
Botulinum toxin injections into specific spastic muscles can provide temporary relief and facilitate participation in therapy
Selective dorsal rhizotomy is a surgical option for severe spasticity, typically in the lower limbs
Other medications may be used for co-existing conditions like epilepsy or reflux.
Surgical Management:
Surgical interventions are considered when conservative management fails to achieve functional goals
These include orthopedic procedures for contractures (e.g., hamstring lengthening, adductor tenotomy), bony deformities (e.g., osteotomies), and hip subluxation
Selective dorsal rhizotomy (SDR) may be considered for significant lower limb spasticity
Tendon transfers can also improve gait mechanics
These are usually performed in conjunction with intensive post-operative physiotherapy and orthotic management.
Supportive Care:
Regular multidisciplinary team meetings involving pediatricians, neurologists, physiatrists, physical therapists, occupational therapists, orthotists, speech therapists, and social workers
Parent education and support are vital
Nutritional assessment and management are important, especially if feeding difficulties are present
Addressing psychosocial aspects and promoting social inclusion.
Complications
Early Complications:
Acute exacerbation of spasticity post-surgery or during illness
Skin breakdown from ill-fitting orthoses
Pain related to spasticity and immobility
Falls due to poor balance and gait instability.
Late Complications:
Progressive joint contractures leading to fixed deformities (e.g., equinus foot, hip dislocation)
Osteoarthritis secondary to malalignment and joint stress
Chronic pain syndromes
Pressure sores
Scoliosis
Respiratory compromise in severe cases due to chest wall deformity and immobility
Social isolation and reduced educational/vocational opportunities.
Prevention Strategies:
Regular monitoring and timely adjustment of orthoses
Consistent adherence to prescribed physical and occupational therapy programs
Early surgical intervention for progressive deformities or contractures
Proactive management of spasticity
Education on proper positioning and skin care
Early identification and management of co-morbidities like epilepsy and dysphagia.
Prognosis
Factors Affecting Prognosis:
Severity of spasticity and neurological impairment
Presence and severity of co-morbidities (e.g., cognitive impairment, epilepsy, visual/auditory deficits)
Age of onset of intervention
Family adherence to therapy and management plan
Quality of multidisciplinary care provided
Extent of preventable secondary complications
Specific lesion location and type on brain imaging.
Outcomes:
With appropriate and consistent management, many children with spastic diplegia can achieve functional ambulation, though often with residual gait deviations
The degree of independence in daily living activities varies widely
Early and intensive intervention often leads to better functional outcomes and reduced long-term disability
Individuals can lead fulfilling lives with appropriate support and accommodations.
Follow Up:
Lifelong follow-up is typically required
Regular reviews by a multidisciplinary team are essential to monitor growth, musculoskeletal development, spasticity, functional status, and to adjust orthotic and therapeutic interventions as needed
Transition to adult healthcare services should be planned well in advance
Continued assessment of educational, vocational, and social needs is important.
Key Points
Exam Focus:
DNB/NEET SS exams often focus on the role of AFOs in gait correction, the principles of gait training in spastic CP, medical management of spasticity (drugs, doses), and indications for surgical interventions like SDR
Understanding the impact of spasticity on motor milestones and the multidisciplinary approach is critical.
Clinical Pearls:
Always assess for hip subluxation/dislocation in children with spastic diplegia, especially if adductor spasticity is significant
The goal of AFOs is not necessarily a "perfect" gait, but a safe, stable, and energy-efficient one that allows for participation
Engage families as active partners in the management plan
their adherence is key to success.
Common Mistakes:
Over-reliance on orthotics without adequate therapy, or vice versa
Underestimating the impact of co-morbidities on functional outcomes
Delaying surgical or orthopaedic interventions for correctable deformities
Inadequate follow-up leading to preventable secondary complications
Failing to tailor management plans to the individual child's needs and goals.